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On April 11, 1890, Joseph Carey Merrick was found dead in his bed at the age of 27. Though the exact cause is unclear, it is believed that he died as a result of the weight of his head, due either to spinal dislocation or asphyxiation from pressure on his airway. Measured by doctors at the time as having a circumference of 36 inches, Merrick’s malformed head was one of many deformities that people came to gape at as he toured England as a human oddity.

Describing by biographers as an avid writer of letters, little of his correspondence appears available to public view. One remaining letter thanks a friend for sending some grouse and a book, the former described as “splendid.” He often concluded with a poem by hymnist Isaac Watts called “False Greatness,” which begins with the lines:

'Tis true my form is something odd, But blaming me is blaming God

In addition to his cranial deformity, Merrick also suffered from grayish skin growths, a curved spine, and a severely enlarged right arm and hand. Though DNA studies on his skeleton have been undertaken to ascertain the exact cause of these abnormalities, the work has been stymied by the repeated bleaching the bones have undergone.

Various diagnoses have been theorized, starting with Merrick’s own folk belief that the problems were due to his mother being frightened by an elephant when she was pregnant with him. While that cause may be safely discarded, the correct one is hard to pin down.

I recall being taught during my medical education that a likely diagnosis was neurofibromatosis type 1. A disease typified by tumors arising from nerve tissue, those with neurofibromatosis can develop skin lesions, spinal curvatures and large heads. During my pediatric residency I was involved in a very sad case of a severely affected young man, who had a large cranial mass that gave him an appearance similar to Merrick’s. That they shared a diagnosis seemed quite plausible to me.

This growth is due to spontaneous mutations in a specific gene, and the severity of the disease depends on when the mutation occurs during embryonic development. Those with the disease have some cells that are genetically normal and some with the mutation. This discordant growth certainly sounds consistent with Merrick’s condition.

After police closed the exhibit in which Merrick was put on public display, his circumstances grew progressively more dire. He was beaten and robbed when sent to tour Europe, after which he made his way back to England. His predicament eventually become something of a cause célèbre, attracting even the attention of the Princess of Wales. Over time he gained enough support to live under Dr. Treves’ care in a London hospital, where he received visitors, went to the theater, and composed poetry.

The exact circumstances of his death are not clear. He slept in an upright position in a custom armchair, so the reasons for his lying down to sleep are open to speculation. At the end of “The Elephant Man,” it is presented as a conscious, life-ending decision, but as Merrick was alone when it happened that is more poetic license than verifiable fact. What is known is that his disease continued to progress over time, requiring more and more care from nurses under the guidance of Dr. Treves at the Royal London Hospital.

The gentle, erudite soul within a body the public considered an oddity is the contrast at the heart of “The Elephant Man.” This theme is enjoying particular cultural resonance of late, explored (albeit in a much more grisly, less realistic manner) in the latest iteration of “American Horror Story.” Embodying both the disfigured exterior and the sensitive man inside is the challenge facing Cooper.

Daniel Day-Lewis enacted a similarly dramatic change when he starred in “My Left Foot,” playing a writer afflicted with severe cerebral palsy; it won him the first of his three (and counting) Academy Awards. Philip Anglim, who starred in the original Broadway cast of “The Elephant Man,” received a nomination for Best Actor at 1979’s Tony Awards, an accomplishment Cooper would doubtless be glad to duplicate (hopefully going home with the trophy this time around).

The cause of Merrick’s physical deterioration and suffering remains a subject of speculation and study, though DNA studies may yet lead to a definitive diagnosis. Whatever the cause, his legacy is that of an intelligent, literate man considered little more than a freak by those who saw only the ravages disease had taken on his body. How effectively Cooper captures both the frailty of that misshapen body and the delicacy of the soul within remains for opening night audiences to see.